Serum Carnitine Levels Research Articles

Correlation between serum carnitine level and Soluble Receptors for Advance Glycation End Products(sRAGE) in Clomiphene Citrate Resistant- PCOS Women

The most frequently diagnosed condition in women at the age of reproduction is the polycystic ovarian syndrome (PCOS).it could be related to a complex endocrine condition, due to its heterogeneity and uncertainty about its etiology, as the clinical highlights of PCOS incorporate those related to reproductive signs such as decreased frequency of ovulation, irregular menstrual cycles, decreased fertility. Carnitine plays a substantial role in weight loss, glucose tolerance, insulin function and fatty acid metabolism. Thus carnitine plays a crucial role in controlling obesity, insulin resistance, oxidative stress that are associated with PCOS .While, AGEs are a diverse group of reactive molecules that are formed endogenously by non-enzymatic reactions of carbonyl group of carbohydrates with free amino groups of proteins, nucleic acids or lipids. The soluble form of receptors of AGE (sRAGE) could play an important role in management obesity, insulin resistance, hyperandrogenism, oxidative stress which could be related to PCOS. This study was aimed to investigate serum levels of carnitine & soluble receptors for advanced glycation end products (sRAGE) in clomiphene resistant PCOS .Besides assessing the correlation between serum levels of carnitine ,as well as, soluble receptors of AGE with hormonal ( LH, FSH& Testosterone) and metabolic ( serum glucose , serum insulin & HOMA-IR) markers in these patients .
 The study included thirty women with clomiphene resistant PCOS and thirty apparently healthy women, as a control .In order to measure serum total carnitine and serum soluble receptor of advance glycation end product (sRAGE in PCOS and control groups.
 The results of our study have shown a decreased serum levels of total carnitine in PCOS group in comparison with control group (48.05 and 59.73 nmol/ml, respectively), but there was no significant elevation in serum levels of sRAGE in patients group as compared with control group .In addition to a significant correlation between serum total carnitine and serum sRAGE levels (r=0.45, P-value=0.03). 
 In conclusion serum total carnitine level was low in Clomiphene resistant-PCOS patients in comparison with control group. Although, sRAGE levels in clomiphene resistant- PCOS patients were not significantly different from the age and BMI-matching controls, but a significant correlation between serum total carnitine and sRAGE was detected.
 Key words: Poly cystic ovarian syndrome, soluble receptor of advance glycation end products (sRAGE) , SerumTotal Carnitine

The Association Between Serum Carnitine Level, Glucose Regulation, Body Fat and Nutrient Intake in Diabetic Individuals

Abstract Carnitine (β-hydroxy-γ-trimethyl amino butyrate) is, a vitamin-like substance carrying long-chain fatty acids into the mitochondrial matrix. Due to its effect in energy metabolism, carnitine plays an important role in controlling diabetes and its complications. Studies on this topic have often focused on carnitine supplementation. This study was planned to investigate the relationship between serum carnitine level, glucose regulation and body fat in diabetic patients. A total of 64 people between the ages of 30-5, 32 patients with type 2 diabetes and 32 healthy subjects, were included in the study. Individual lipid profiles, glucose, insulin and serum carnitine levels were analyzed, anthropometric measurements were taken and 24-hour recall food consumption was recorded. As a result, blood glucose, insulin, triglyceride, VLDL-C, HDL-C and HOMA-IR were found to be higher in diabetic individuals than healthy group (p<0,05). Serum carnitine levels were found to be significantly lower in diabetic male (50,6±20,83 nmol/mL) than in healthy male (59,5±17,25 nmol/mL)(p<0,05). This difference was not statistically significant among female (p>0,05). It has been observed that intake of energy and macronutrients of diabetic individuals is generally lower than that of healthy individuals. Serum carnitine level was positively associated with polyunsaturated fatty acids and omega-6 fatty acid intake in male in the healthy group showed a negative correlation with fiber intake in female in the healthy group (p<0,05). There were negative correlations between serum carnitine level with body weight, body mass index and body fat mass in female in the healthy group (p<0,05). Individuals with diabetes are predisposed to dyslipidemia and insulin resistance. As a result; food consumption, and body fat affect individuals’ serum carnitine levels in type-2 diabetes. Since there is not enough study evaluating the relationship between anthropometric measurements of individuals and serum carnitine levels, it is thought that this result will guide future studies.

Significance of Levocarnitine Treatment in Dialysis Patients.

Carnitine is a naturally occurring amino acid derivative that is involved in the transport of long-chain fatty acids to the mitochondrial matrix. There, these substrates undergo β-oxidation, producing energy. The major sources of carnitine are dietary intake, although carnitine is also endogenously synthesized in the liver and kidney. However, in patients on dialysis, serum carnitine levels progressively fall due to restricted dietary intake and deprivation of endogenous synthesis in the kidney. Furthermore, serum-free carnitine is removed by hemodialysis treatment because the molecular weight of carnitine is small (161 Da) and its protein binding rates are very low. Therefore, the dialysis procedure is a major cause of carnitine deficiency in patients undergoing hemodialysis. This deficiency may contribute to several clinical disorders in such patients. Symptoms of dialysis-related carnitine deficiency include erythropoiesis-stimulating agent-resistant anemia, myopathy, muscle weakness, and intradialytic muscle cramps and hypotension. However, levocarnitine administration might replenish the free carnitine and help to increase carnitine levels in muscle. This article reviews the previous research into levocarnitine therapy in patients on maintenance dialysis for the treatment of renal anemia, cardiac dysfunction, dyslipidemia, and muscle and dialytic symptoms, and it examines the efficacy of the therapeutic approach and related issues.

Hypoglycemia Due to Acquired Carnitine Deficiency in a Pediatric Patient Receiving Chemotherapy.

We describe a 21-month-old male with relapsed clear cell sarcoma of the kidney receiving enteral nutrition who experienced recurrent, ketotic hypoglycemia. During relapse therapy, he had recurrent hypoglycemia episodes, in the setting of hematochezia and diarrhea. Evaluation revealed low carnitine levels. He received supplementation with oral levocarnitine throughout the remainder of treatment, resulting in normalization of serum carnitine levels and no further hypoglycemia. We believe adverse effects of the chemotherapy on his single kidney and gastrointestinal insult resulted in hypoglycemia and carnitine deficiency. Our case highlights that carnitine deficiency should be considered when acute onset hypoglycemia without obvious cause occurs.

Effectiveness of monitoring free carnitine levels for L-carnitine supplementation in hemodialysis patients to maintain carnitine sufficiency and nutritional factors.

We investigated the effectiveness of monitoring serum carnitine levels in hemodialysis patients receiving L-carnitine supplementation. One-hundred forty-five hemodialysis patients were divided into three groups. Group 1 consisted of patients (n= 30) who had been receiving supplementation before this study and then discontinued at the beginning. The remaining patients were divided into Group 2 (n= 13) and Group 3 (n= 102) based on their baseline free carnitine (FC) level, <20 or ≥ 20 μmol/L. Group 2 was started on supplementation, and Groups 1 and 3 were observed without any intervention for the first 6 months. FC was measured every 6 months in all three groups up to 18 months. All patients in whom FC was less than 20 μmol/L at 6 and 12 months were prescribed supplementation. After the first 6 months, the mean ± SD of FC changed from 262.5 ± 87.5 μmol/L at baseline to 70.8 ± 33.6 μmol/L (P< 0.001) in Group 1, from 17.4 ± 1.9 to 193.9 ± 43.3 μmol/L (P< 0.001) in Group 2, and from 49.2 ± 24.6 to 44.2 ± 19.8 μmol/L (P< 0.05) in Group 3. The acyl/free carnitine changed from 0.62 to 0.59 in Group 1 (P= 0.287), from 0.76 to 0.66 in Group 2 (P= 0.054) and from 0.57 to 0.60 in Group 3 (P< 0.05). Of the 145 patients, 126 continued follow-up for the full 18 months. FC remained in the normal range (36-74 μmol/L) within the 95% CI. FC was considered a strong predictor of carnitine deficiency after 6 months (AUC: 0.9146, cut-off value: 33.8 μmol/L). FC monitoring is essential for appropriate carnitine supplementation in hemodialysis patients.

The correlation between carbamazepine and valproic acid monotherapy with serum adiponectin and carnitine

Antiepileptic drugs may cause systemic and metabolic side effects. The aim of our study was to investigate the effects of valproic acid and carbamazepine monotherapy used in the treatment of epilepsy patients on serum adiponectin and carnitine levels. The study included 60 patients, of which 30 patients receiving valproic acid monotherapy and 30 patients receiving carbamazepine monotherapy, who were followed up by the epilepsy outpatient clinic with the diagnosis of idiopathic epilepsy, and 30 healthy volunteers. Patients, who used drugs for at least 6months, were selected. Venous blood samples were collected from the patients and healthy volunteers after their consent was obtained. Serum carnitine and adiponectin levels in the collected samples were measured using the ELISA method. Serum carnitine levels were 5166.55ng/ml (± 1954.92) in patients receiving carbamazepine, 4224.56ng/ml (± 2055.54) in patients using valproic acid, and 5802.64ng/ml (± 3422.57) in the control group. Serum adiponectin levels were 13,606.51ng/ml (± 5915.92) in patients using carbamazepine, 11,986.58ng/ml (± 5367.82) in patients receiving valproic acid, and 14,033.43ng/ml (± 5646.34) in the control group. In both groups, both serum carnitine and serum adiponectin levels were lower than the control group. There was a negative but insignificant correlation between the duration and dose of carbamazepine and valproic acid drug use and serum adiponectin and carnitine levels. There is a need for more extensive studies with larger sample size to investigate the effect of antiepileptic drugs used on serum adiponectin and carnitine levels.

Evaluation of Serum Level of Carnitine in Children with Acute Pyelonephritis (APN) Compared to Healthy Children.

This cross-sectional case-control study evaluated the serum carnitine level in children with urinary tract infection (UTI). Acute pyelonephritis (APN) is a common bacterial infection of the upper urinary tract in children which may also lead to renal damage and tubular atrophy. Activation of inflammatory mediator bedside alterations in the cytokines and generation of reactive oxygen species (ROS) play a striking role in the development of tissue damage after pyelonephritis. L-carnitine as one of the most potent natural antioxidant agents by inhibition of lipid peroxidation may protect cells and tissues from damage. A total of 30 children with UTI (as a case group) and 30 healthy children (as a control group) which matched in terms of age and sex were enrolled in this study. All children were evaluated and compared with respect to age, sex, weight, body mass index (BMI) and serum carnitine level. Serum carnitine level was determined using serum carnitine ELISA kit. Demographic and clinical data such as age, sex, weight and BMI were not statistically significant between the two groups. The serum carnitine levels were significantly lower in the case group with UTI than the control group. Mean serum carnitine concentration in the case group and in the control group was 36.56 ± 9.87 μmol/l and 62.8±21.35, respectively (P = 0.001). According to our study, it could be concluded that low serum L-carnitine level is linked to UTI in children. Therefore, further studies are needed to confirm our results.

Impact of Serum level of L-Carnitine on Chitotriosidase in Women with Gestational Diabetes Compared to T2DM Pregnant &amp; Non Pregnant Iraqi Women

Background: Gestational diabetes (GDM) was defined as any degree of glucose intolerance that to be firstrecognized during pregnancy, regardless of the degree of high blood sugar. L-Carnitine appeared to act as avitamin and thus termed as vitamin BT. Under certain conditions, the demand for carnitine may exceed anindividual’s capacity to synthesize it, making it a conditionally essential nutrient. Whereas, chitotriosidase ischitinase family member enzyme, produced by activated macro phages. Enzymatic activity of chitotriosidaseincreases significantly during the development of acute or chronic disorders.Aim: The study involved estimating serum level of L-carnitine &amp; activity of chitotriosidase in womenwith gestational diabetes compared to type-2 diabetic pregnant &amp; non pregnant Iraqi women. In addition toassessing the possible impact of variations of serum level of carnitine on serum chitotriosidase activity inthe study groups.Methodology: This is a case control study included seventy-five women whom attending the Al AlwaiyaMaternity Teaching Hospital/Baghdad-Iraq. The participants were selected of similar age consisted of threegroups: 25 pregnant women with gestational diabetes, 25 pregnant women with type 2 diabetes, and 25healthy non pregnant women as a control. After the provision of the ethical approval from Ethics Committeeof the Collage of Pharmacy–University of Baghdad. The studied biochemical markers were: total HbA1c,lipid profile, serumL-carnitine &amp; chitotriosidase.Results and Discussion: Data analysis for comparing the groups results (gestational diabetes, T2DM andcontrol) indicated significant variations among them considering serum carnitinelevels &amp; chitotriosidaseactivity. However, HbA1C levels were positively correlated with L-carnitineamong GDM pregnantwomen, with highest levels compared to the control &amp; T2DM pregnant women. However, serum carnitinelevels were significantly correlated with chitotriosidase activity among GDM pregnants specifically (r=-0.813,p=0.0001).Conclusion: Serum carnitine level was significantly lowered in T2DM pregnant women,as well as in pregnantwomen with GDM, as being compared to healthy non pregnant women. Whereas, serum chitotriosidaselevel were significantly elevated in women with GDM, in comparison to T2DM and controls, it’s activity isnegatively correlated with serum carnitine level.

Study of the Relationship Between Serum Amino Acid Metabolism and Lymph Node Metastasis in Patients with Colorectal Cancer.

PurposeLymph node metastasis is one of the important prognostic factors of colorectal cancer, and an important index of individualized treatment. The purpose of this study is to use metabonomics to identify potential molecular markers of lymph node metastasis in colorectal cancer (CRC).Patients and MethodsPeripheral blood samples of 223 CRC patients were collected. The metabolic levels of amino acids and carnitine in peripheral blood of CRC patients, with and without lymph node metastasis, were analyzed by liquid chromatography-tandem mass spectrometry (LC-MS/MS).ResultsThe results show that there were significant differences in the levels of serum amino acids and carnitine between lymph node metastatic patients and lymph node non-metastatic patients. The diagnostic model that was constructed by 9 types of serum metabolites has a high diagnostic ability.ConclusionLC-MS/MS is a detection method that has a broad application in predicting CRC prognosis, individualized treatment, and in studying the mechanism of lymph node metastasis.

Sorafenib Might Induce Sarcopenia in Patients With Hepatocellular Carcinoma by Inhibiting Carnitine Absorption.

Tyrosine kinase inhibitors (TKIs) have revolutionized the treatment of cancers. Sorafenib, an oral multi-target TKI, improves the median overall survival time in patients with hepatocellular carcinoma (HCC). It also inhibits the absorption of carnitine by down-regulating the human organic cationic transporter OCTN2 located largely in the small intestinal mucosa and skeletal muscle. The aim of the study was to determine, by assessing carnitine metabolism, whether sarcopenia is induced in patients with HCC who are receiving sorafenib. This retrospective study included 110 adult Japanese patients with liver cirrhosis and HCC who received sorafenib. Sorafenib was administered at a dose of 200-800 mg/day for 4 weeks. Blood samples were collected before and after treatment, and serum carnitine fraction and myostatin levels were measured. Cross-sectional areas (cm2) of the skeletal muscles at the third lumbar vertebra level were determined by manually outlining computed tomography images before and after treatment. The cross-sectional areas were normalized for height [skeletal muscle index (SMI), cm2/m2]. Patients were allocated to two groups according to Child-Pugh (CP) class; 81 had CP-A liver function, and 29 had CP-B. SMI after treatment was significantly lower than that before treatment in both groups. Serum levels of total carnitine and free carnitine after treatment were significantly lower than those before treatment in both groups. There were no differences in serum levels of myostatin before and after treatment in either group. Sorafenib might decrease serum levels of carnitine by inhibiting carnitine absorption. Decreasing of serum levels of carnitine might lead to presarcopenia.

Hepcidin and its Related Hematological Biomarkers of Anemia in Children on Hemodialysis: Role of Carnitine Deficiency

BACKGROUND: Anemia is one of the most common complications in end-stage renal disease (ESRD) patients. Hepcidin is a hormone that regulates iron homeostasis in patients with ESRD. Carnitine deficiency is commonly seen in hemodialysis (HD) patients.&#x0D; AIM: This study aimed to investigate the relationship between hepcidin and inflammatory and other anemia markers in children with ESRD and to evaluate the association of carnitine deficiency with anemia in these patients.&#x0D; SUBJECTS AND METHODS: Thirty pediatric patients with ESRD undergoing HD, and thirty healthy, age- and sex-matched children served as controls were included in the study. Serum levels hepcidin, iron status, high-sensitivity C-reactive protein, and total carnitine were measured.&#x0D; RESULTS: Statistically significant increases in serum levels of hepcidin (100.7 ± 0.99 ng\ml vs. 77.43 ± 0.8 ng\ml, p = 0.000), was found in HD children as compared to healthy controls. Statistically significant increase in serum levels of hs-CRP (3.94 ± 0.19 mg/l vs. 1.36 ± 0.07 mg/l, p = 0.04) was found in HD children as compared to healthy controls. However, serum levels of carnitine (29.59 ± 2.46 μmol/L vs. 36 ± 2.39 μmol/L, p = 0.000) showed statistically significant decreases in HD children as compared to healthy controls positive correlation was found between hepcidin and hs-CRP (r = 0.059, p = 0.042). Furthermore, a positive correlation was present between serum carnitine levels and serum iron levels (r = 0.651, p = 0.042).&#x0D; CONCLUSION: Serum hepcidin may be a more useful biomarker of functional iron deficiency in children on HD. The efficacy of carnitine treatment for children on HD with carnitine deficiency and its effect on anemia needs to be studied.

Association Between the Serum Carnitine Level and Ammonia and Valproic Acid Levels in Patients with Bipolar Disorder.

Valproic acid (VPA) is not only an antiepileptic drug but also a mood stabilizer for patients with bipolar disorder. Long-term VPA therapy can cause carnitine deficiency, which may result in an increase in the blood ammonia level, in patients with epilepsy. However, information about this effect in patients with bipolar disorder is limited. The aim of this study was to investigate the associations between the serum VPA level and the carnitine and ammonia levels in psychiatric adult patients with epilepsy. The subjects were 182 consecutive Japanese adult patients (mean age 54.3 ± 19.5 years) diagnosed with bipolar disorder and treated with VPA. The serum VPA level, carnitine fraction, and plasma ammonia level were measured. Furthermore, the free carnitine and acylcarnitine fractions were measured using an enzyme cycling method. Sixty-nine patients (38%) had a low free carnitine level. There were significant differences in sex, height, VPA dose, serum VPA level, total carnitine level, acylcarnitine level, and acylcarnitine/free carnitine ratio between patients with a low free carnitine level and those with a normal range of free carnitine. The simple and multiple regression analyses revealed that the VPA dose and the serum VPA level were inversely and significantly correlated with the free carnitine level. The plasma ammonia level was correlated with the VPA dose, serum VPA level, and acylcarnitine level but not with the free carnitine level. These findings suggest that carnitine deficiency is associated with the VPA dose and the serum VPA level in patients with bipolar disorder. However, it is unlikely that carnitine deficiency is associated with hyperammonemia in patients with bipolar disorder.

The Effect of Carnitine Supplementation on Left Ventricular Function: Lessons from Current Evidence and Insights for Future Studies

Introduction: In children, data on the effects on carnitine supplementation and myocardial function are limited. A few studies have investigated the relationship between serum carnitine levels in the setting of depressed cardiac function and have demonstrated possible benefits. As such, this systematic review and meta-analyses aimed to assess the effects carnitine supplementation on left ventricular function. Materials and Methods: A systematic review of the literature was performed to identify full text manuscripts in English. PubMed, EMBASE, and the Cochrane databases were queried. Studies were included with data from pediatric patients, that used carnitine supplementation and included preand post-carnitine data for at least one of the outcomes of interest. Results: A total of six studies including 144 patients were included. Carnitine dosage ranged from 50 to 100 mg/kg/day. The average duration of carnitine therapy was 9.8 months. Left ventricular ejection fraction was higher after carnitine supplementation with a mean difference between groups of 3.56 [95% confidence interval 0.06–7.06, p-value 0.04]. Left ventricular shortening fraction was higher after carnitine supplementation with a mean difference between groups of 3.68 [95% confidence interval 1.22–6.15, p-value 0.01]. Left ventricular end diastolic diameter was higher after carnitine supplementation, but the difference did not reach significance. Conclusion: Carnitine supplementation may augment left ventricular ejection fraction and shortening fraction. Those with lower baseline ejection fraction and shortening fraction appear to benefit the most from carnitine supplementation. Additional studies of the effects of carnitine on cardiac function are warranted.

Kinetics of Serum Carnitine Fractions in Patients with Chronic Kidney Disease Not on Dialysis.

Carnitine plays a pivotal role in energy synthesis through β-oxidation in mitochondria. Serum and tissue levels of free carnitine are significantly decreased in dialysis patients, whereas acylcarnitine levels are increased. However, the precise kinetics and fate of carnitine fractions in chronic kidney disease (CKD) patients who are not on dialysis have not been clarified. This study aims to determine the kinetics of serum carnitine fractions in patients who were not on dialysis. Seventy-five CKD patients not on dialysis were recruited in this study. Serum and urinary carnitine fraction levels were measured to evaluate the kinetics and regulation of serum carnitine fractions. Carnitine fractions were measured by the enzymatic cycling method. Total and free serum carnitine levels did not change with progression of CKD, whereas acylcarnitine levels and the acyl/free carnitine ratio significantly increased. Serum acylcarnitine levels were inversely associated with estimated glomerular filtration rate (r2 = 0.239, p < 0.001), but free carnitine levels were not. Serum free carnitine levels were positively associated with urinary free carnitine excretion (r2 = 0.214, p < 0.001), but serum acylcarnitine levels were not. Multiple stepwise regression analysis revealed that urinary free carnitine excretion and blood urea nitrogen were independent determinants of serum free carnitine and acylcarnitine levels, respectively. The present study demonstrated that serum acylcarnitine levels increased with renal dysfunction independent of urinary excretion levels. Serum free carnitine was not affected by renal function in CKD patients who were not on dialysis.

First Case Report of Primary Carnitine Deficiency Manifested as Intellectual Disability and Autism Spectrum Disorder

Systemic primary carnitine deficiency (PCD) is a genetic disorder caused by decreased or absent organic cation transporter type 2 (OCTN2) carnitine transporter activity, resulting in low serum carnitine levels and decreased carnitine accumulation inside cells. In early life, PCD is usually diagnosed as a metabolic decompensation, presenting as hypoketotic hypoglycemia, Reye syndrome, or sudden infant death; in childhood, PCD presents with skeletal or cardiac myopathy. However, the clinical presentation of PCD characterized by autism spectrum disorder (ASD) with intellectual disability (ID) has seldom been reported in the literature. In this report, we describe the clinical features of a seven-year-old girl diagnosed with PCD who presented atypical features of the disease, including a developmental delay involving language skills, concentration, and attention span, as well as autistic features and brain alterations apparent in magnetic resonance imaging. We aim to highlight the difficulties related to the diagnostic and therapeutic approaches used to diagnose such patients. The case reported here presented typical signs of PCD, including frequent episodes of hypoglycemia, generalized muscle weakness, decreased muscle mass, and physical growth deficits. A molecular genetic study confirmed the definitive diagnosis of the disease (c.1345T>G (p.Y449D)) in gene SLC22A5, located in exon 8. PCD can be accompanied by less common clinical signs, which may delay its diagnosis because the resulting global clinical picture can closely resemble other metabolic disorders. In this case, the patient was prescribed a carnitine-enriched diet, as well as oral carnitine at a dose of 100 mg/kg/day. PCD has a better prognosis if it is diagnosed and treated early; however, a high level of clinical suspicion is required for its timely and accurate diagnosis.

Serum carnitine levels of children with epilepsy: Related factors including valproate

ObjectiveThis study measured the serum carnitine levels in patients with epilepsy and determined the factors contributing to low carnitine levels. MethodsWe measured the serum carnitine levels in 94 consecutive patients with epilepsy, including the free carnitine (FC) and acylcarnitine fractions, using an enzyme cycling method. We defined a low FC as a serum FC level < 36 μmol/L. Age, body mass index (BMI), standard deviation score of BMI (BMI-SDS), use of valproate, cognitive disorder, and feeding problems differed between patients with low and normal FC. In patients taking valproate, the associations of the serum FC level with the platelet count and serum ammonia and amylase levels were analyzed. ResultsUnivariate analysis showed that a low BMI and BMI-SDS, the use of valproate, and cognitive disorder were more frequent in patients with a low FC. Logistic regression analysis revealed that a low BMI-SDS and cognitive disorders were independently associated with a low FC. Among the patients taking valproate, a low BMI-SDS and age were associated with a low FC. The serum FC and ammonia levels were inversely correlated, whereas no correlation was observed between the serum FC level and platelet count or serum amylase level. ConclusionA low BMI and cognitive disorders were related to a low FC in patients with epilepsy and the serum carnitine levels should be monitored in these patients.

Study of serum and seminal carnitine levels in Egyptian men with chronic hepatitis C

Objective The aim of the study was to evaluate the serum and seminal carnitine levels in Egyptian men with chronic hepatitis C virus (HCV) infection. Background In 2015, HCV still affects a substantial proportion of the Egyptian population, in the 1–59 year age, 5.3 million persons are positive for HCV antibodies. Numerous studies have investigated the benefits of carnitine supplementation on improving the complications of viral hepatitis, like steatosis and hepatic encephalopathy. Patients and methods A cross-sectional study was conducted on 35 patients with HCV attending the hepatitis outpatient clinic, in Shebin El-Kom Teaching Hospital, Egypt. Moreover, 20 healthy individuals were also included as a control group. They were all subjected to history taking and serum and semen carnitine level examination. Results Results revealed an insignificant difference between patients with HCV and healthy individuals concerning age, residence, and serum urea level. However, a significant difference was found between the groups regarding serum glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, creatinine level, prothrombin activity (%), and serum and semen carnitine levels. Moreover, a nonsignificant positive correlation (r = 0.331, P = 0.052) was founded between serum and semen carnitine levels in patients with HCV; in addition, a nonsignificant negative correlation (r = −0.364, P = 0.301) was found between serum and semen carnitine levels in the control group. Conclusion Results concluded that patients with HCV had significantly higher serum glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, urea, and creatinine level. However, they had significantly lower prothrombin activity and serum and semen carnitine levels compared with the healthy group. Moreover, an insignificant correlation was detected between serum and semen carnitine levels for patients with HCV. So, many studies on various world population with this trend are warranted.

Carnitine deficiency in epileptic children treated with a diversity of anti-epileptic regimens

BackgroundCarnitine deficiency is relatively common in epileptic patients. The risk factors reported include the combination of valproic acid with other antiepileptic drugs (AEDs), young age, multiple neurologic disabilities, non-ambulatory status, and being underweight.ObjectivesTo study the level of carnitine deficiency and its associated risk factors among a group of children with idiopathic epilepsy treated with different AEDs.Patients and methodsFifty children with idiopathic epilepsy and 40 age-matched controls were enrolled. For all, serum carnitine level was measured by enzyme-linked immune sorbent assay (ELISA).ResultsThe mean carnitine level was lower in cases compared to controls (p = 0.04). Patients receiving monotherapy treatment had a high percentage of carnitine deficiency compared to controls (p = 0.04). Patients receiving valproate with other AEDs had a lower level of carnitine compared to controls (p = 0.03). The age of the patients, the duration of treatment, and the doses of different AEDs were not risk factors for carnitine deficiency.ConclusionsCarnitine deficiency is common in our population, and the use of valproate with other AEDs is considered the most important risk factor for it in epileptic children.

Renal dysfunction is rare in Fukuyama congenital muscular dystrophy

BackgroundThe leading cause of death in patients with Fukuyama congenital muscular dystrophy (FCMD) is congestive heart failure or respiratory dysfunction, which is same as that in Duchenne muscular dystrophy (DMD). Recent studies reported that renal dysfunction is a common complication and an increasing cause of death in advanced DMD. It can be attributable to circulatory instability or inappropriate use of drugs for treating cardiac dysfunction. MethodsWe retrospectively evaluated renal function in 38 genetically diagnosed patients with FCMD (range, 1.3–32.9 years; mean age, 13.7 ± 6.9 years) using cystatin C. We examined possible relationships of cystatin C with blood natriuretic peptide and creatinine levels along with cardiac echocardiography findings. ResultsTwenty-five patients were treated for cardiac dysfunction. Elevated cystatin C level was detected only in two, who also showed proteinuria, glycosuria, hematuria, and extremely high β2-microglobulin levels on urine tests, and were thus diagnosed with renal tubular cell damage. Because both patients were treated for intractable epilepsy with various antiepileptic drugs, including valproic acid (VPA), and had low serum carnitine levels, renal tubular cell damage was considered as an adverse effect of VPA. Unlike patients with DMD, no patient with FCMD had renal dysfunction. Such a rare occurrence of renal dysfunction can be attributable to mild cardiac dysfunction, short disease duration, and careful and early fluid management. ConclusionRenal dysfunction is rare in patients with FCMD; however, renal tubular cell damage should be ascertained, particularly in those undergoing VPA treatment for epilepsy.

Effect of early supplementation of exogenous carnitine on liver mitochondrial damage in severely scalded rats and its pathological mechanism

Objective: To observe the effect of early supplementation of exogenous carnitine on liver mitochondrial damage in severely scalded rats and to explore its pathological mechanism. Methods: Seventy-two adult female Sprague-Dawley rats were divided into sham injury group, scald injury group, and scald injury+ carnitine group according to the random number table, with 24 rats in each group. Rats in sham injury group was sham injured on the back by immersing in 37 ℃ water bath for 12 s without fluid replacement. While rats in scald injury and scald injury+ carnitine groups were inflicted with 30% total body surface area (TBSA) full-thickness scald on the back by immersing in 98 ℃water bath for 12 s. Immediately after injury, rats in scald injury group and scald injury+ carnitine group were injected with Ringer's lactate solution with the dosage of 4 mL·kg(-1)·%TBSA(-1) via tail vein according to the Parkland formula, meanwhile rats in scald injury+ carnitine group were injected with L-carnitine solution with dosage of 300 mg·kg(-1)·d(-1) via tail vein from post injury hour (PIH) 1. At PIH 12, 24, 48 and 72, abdominal aorta blood and liver tissue were collected from 6 rats in each group. The serum levels of carnitine, β-hydroxybutyric acid, and ornithine carbamoyltransferase (OCT) were determined with enzyme-linked immuno sorbent assay, and the serum levels of lactate dehydrogenase (LDH), alanine aminotransferase(ALT), and aspartate transaminase (AST) was determined by automatic biochemical analyzer, Pathological changes of rats liver tissue were detected with HE staining. Data were processed with analysis of variance of factorial design and Student-Newman-Keulstest or Tamhane test, Bonferroni correction. Results: (1) Compared with sham injury group, the serum level of carnitine of rats in scald injury group was significantly lower at each time point (P 0.05]. The serum level of carnitine in scald injury+ carnitine group was significantly higher than that in scald injury group at each time point (P 0.05). The serum levels of LDH of rats in scald injury+ carnitine group were significantly lower than those of scald injury group at each time point (P 0.05). The serum levels of ALT and AST in scald injury+ carnitine group both showed a trend of decrease, and they peaked at PIH 12 [(260±25), (1 511±145) U/L, respectively]. (6) The liver tissue of rats in sham injury group was basically normal at each time point. The degree of liver injury of rats in scald injury+ carnitine group was lighter than that in scald injury group. The liver tissue of rats in scald injury group at PIH 72 showed obvious cytoplasm loose, liver tissue structure loss with diffuse fatty degeneration and large coagulative necrosis. Only partially scattered fatty degeneration was observed in the liver tissue of ras in scald injury+ carnitine group. Conclusions: By early supplementation of exogenous carnitine, serum levels of carnitine and β-hydroxybutyric acid can be restored to normal levels faster, alleviate mitochondrial damage of hepatocytes, and maintain the metabolic stability of hepatocytes in early stage of severe scald.